WB, W-S, IF-F, IF-IC
H M R
Endogenous
130
Rabbit IgG
#P12109
1291
Product Information
Product Usage Information
| Application | Dilution |
|---|---|
| Western Blotting | 1:1000 |
| Simple Western™ | 1:50 - 1:250 |
| Immunofluorescence (Frozen) | 1:3200 - 1:6400 |
| Immunofluorescence (Immunocytochemistry) | 1:3200 - 1:12800 |
Storage
Specificity / Sensitivity
Species Reactivity:
Human, Mouse, Rat
Source / Purification
Monoclonal antibody is produced by immunizing animals with a synthetic peptide corresponding to residues surrounding Ile536 of human COL6A1 protein.
Background
Collagen type VI (ColVI) is a microfibrillar collagen found in the extracellular matrix (ECM) of muscles, bone, and connective tissues (1). ColVI consists of three main alpha chains: alpha 1, alpha 2, and alpha 3, which are encoded by the COL6A1, COL6A2, and COL6A3 genes, respectively. The three alpha chains form triple helix monomers and tetramers, which further assemble into a beaded microfilament network structure in the ECM (1,2). ColVI interacts with other ECM components, such as collagens, fibronectins, and perlecan, to support ECM mechanical sensing, anchoring the basement membrane to the surrounding ECM and inhibiting apoptosis and oxidative damage (3,4). Mutations in each of the ColVI genes (COL6A1, COL6A2, and COL6A3) result in defective ColVI assembly, causing Ullrich congenital muscular dystrophy (CMD) and Bethlem myopathy due to malformation of ECM structure (5,6). Knockout of COL6A1 in mice displays a mild myopathy and neurodegeneration associated with mitochondrial dysfunction, defective autophagy, and spontaneous apoptosis of muscle fibers (7,8). Increased COL6A1 in the ECM promotes tumor growth, metastasis, and therapeutic drug resistance (9-11).
- Di Martino, A. et al. (2023) Int J Mol Sci 24, 5095. doi: 10.3390/ijms24065095.
- Cescon, M. et al. (2015) J Cell Sci 128, 3525-31.
- Knupp, C. et al. (2006) J Struct Biol 154, 312-26.
- Lamandé, S.R. and Bateman, J.F. (2018) Matrix Biol 71-72, 348-367.
- Pepe, G. et al. (2006) Ann Neurol 59, 190-5.
- Bönnemann, C.G. (2011) Nat Rev Neurol 7, 379-90.
- Cescon, M. et al. (2016) Aging (Albany NY) 8, 1083-101.
- Irwin, W.A. et al. (2003) Nat Genet 35, 367-71.
- Owusu-Ansah, K.G. et al. (2019) Int J Oncol 55, 391-404.
- Zhu, Y.P. et al. (2015) Oncotarget 6, 14488-96.
- Cescon, M. et al. (2023) Cell Mol Life Sci 80, 233.
Species Reactivity
Species reactivity is determined by testing in at least one approved application (e.g., western blot).
Western Blot Buffer
IMPORTANT: For western blots, incubate membrane with diluted primary antibody in 5% w/v BSA, 1X TBS, 0.1% Tween® 20 at 4°C with gentle shaking, overnight.
Applications Key
WB: Western Blotting W-S: Simple Western™ IF-F: Immunofluorescence (Frozen) IF-IC: Immunofluorescence (Immunocytochemistry)
Cross-Reactivity Key
H: human M: mouse R: rat Hm: hamster Mk: monkey Vir: virus Mi: mink C: chicken Dm: D. melanogaster X: Xenopus Z: zebrafish B: bovine Dg: dog Pg: pig Sc: S. cerevisiae Ce: C. elegans Hr: horse GP: Guinea Pig Rab: rabbit All: all species expected
Trademarks and Patents
限制使用
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